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HEM TEST 2024 > PRCA facts > Flashcards

PRCA facts Flashcards

1
Q

Transient aplastic crisis

A

Parvo B19
Predisposing factors of immunodeficiency (HIV/SCID) or hereditary hemolytic anemia (Spherocytosis…)

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2
Q

Diamond- Blackfan anemia

A

Hereditary PRCA

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3
Q

Good syndrome

A

Hypoglobulinemia and thymoma

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4
Q

Diseases associated with PRCA

A

T-LGL
Lymophoma (CLL)
Thymoma
Good syndrome
Post ABO incompatible all transplant

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5
Q

BM diagnosis of PRCA

A

erythroid hypoplasia(>1-5%)

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6
Q

DD of PRCA

A

Copper deficiency
MDS
AA

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7
Q

w/u of PRCA

A

Anemiogram including EPO and Coombs
Erytorocyte adenosine deaminase and genetic testing for Diamond-Blackfan anemia
FACS, PCR TCR and STAT3 mutation for LGL
ANA/RF
PEP/IEP/FLC
Parvo, HBV, HCV, HIV, EBV, CMV
BMB
CT scan of chest for thymoma

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8
Q

Testing for Parvo B19

A

PCR
IgM may aid with Dx
IgG excludes Dx except in context of HIV/SCID

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9
Q

1st line Tx of PRCA

A

CsA +- Steroids

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10
Q

Time to response in PRCA

A

Usually 2-3 months after initiation of Tx

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11
Q

Duration of Tx in PRCA

A

1-2 months after normalization of Hb

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12
Q

CsA effectiveness in PRCA

A

70-80% ORR

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13
Q

Maintenance Tx in PRCA

A

low dose CsA
Combination with Steroids improves relapse free survival

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14
Q

1st line Tx of PRCA when CsA contraindications

A

Cyclophosphamide or MTX +- Steroids

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15
Q

CY effectiveness in PRCA

A

40-60%
median time of 12 weeks
Tx not exceeding 6 months
mainly effective in LGL associated PRCA

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16
Q

MTX in T-LGL associated PRCA

A

Less effective than CY
causes anemia
Mainly effective in T-LGL with neutropenia

17
Q

Thymoma frequency in PRCA

A

30%

18
Q

PRCA frequency in thymoma

A

5-7%

19
Q

2nd line Tx for PRCA

A

Alemtuzumab
Anti-CD52
low dose of 10mg for 10 days or 10 mg 1/w for 4/8 weeks
PCP HSV prophylaxis
IVIG before Tx if hypogamma

20
Q

2nd line Tx for PRCA- other options

A

Sirolimus
ORR 80% in 2nd line
ORR 90% in 1st line (eg. CKD pts)

21
Q

Tx of PRCA in RA pts

A

Tofacitinib

22
Q

Tx of PRCA in CLL pts

A

Rituximab

23
Q

Tx of PRCA in MGUS pts

A

Bortezomib

24
Q
A

HEM TEST 2024 (32 decks)

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